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- Title
Citric acid as the last therapeutic approach in an acute life-threatening metabolic decompensation of propionic acidaemia.
- Authors
Siekmeyer, Manuela; Petzold-Quinque, Stefanie; Terpe, Friederike; Beblo, Skadi; Gebhardt, Rolf; Schlensog-Schuster, Franziska; Kiess, Wieland; Siekmeyer, Werner
- Abstract
The tricarboxylic acid (TCA) cycle represents the key enzymatic steps in cellular energy metabolism. Once the TCA cycle is impaired in case of inherited metabolic disorders, life-threatening episodes of metabolic decompensation and severe organ failure can arise. We present the case of a 6 ½-year-old girl with propionic acidaemia during an episode of acute life-threatening metabolic decompensation and severe lactic acidosis. Citric acid given as an oral formulation showed the potential to sustain the TCA cycle flux. This therapeutic approach may become a treatment option in a situation of acute metabolic crisis, possibly preventing severe disturbance of energy metabolism.
- Publication
Journal of Pediatric Endocrinology & Metabolism, 2013, Vol 26, Issue 5/6, p569
- ISSN
0334-018X
- Publication type
Article
- DOI
10.1515/jpem-2012-0148